Acute Aortic Syndrome 

Acute Aortic Syndrome – Urgent Action

• Obtain emergent surgical consultation for type A dissection; expedite transfer to higher-level facility if indicated ¹
• Decrease stress on aortic wall by decreasing heart rate and blood pressure
  • Begin IV β-blocker to decrease heart rate to 60 beats per minute or less; add nondihydropyridine calcium channel blocker if needed to reach this goal
  • After heart rate is controlled, begin vasodilators to decrease systolic blood pressure to 120 mm Hg or less; nitroprusside is commonly used
  • Administer IV narcotic analgesic for pain

8 Interesting Facts

1. Acute aortic syndrome comprises several life-threatening aortic conditions that present similarly but have differing pathologic, demographic, and survival characteristics; it includes aortic dissection, intramural hemorrhage, and penetrating atherosclerotic ulcer
2. Male individuals older than 40 years with hypertension and individuals younger than 40 years with Marfan syndrome or bicuspid aortic valve are at highest risk
3. Characteristic symptoms include very abrupt onset of chest pain, usually described as sharp, tearing, or ripping. Pain may radiate to neck, back, or abdomen, depending on location of aortic damage
4. CT angiography is usually the diagnostic test of choice, although transesophageal echocardiography or MRI can be used depending on patient characteristics and facility capabilities
5. Initial management includes immediate surgical consultation and urgent reduction of heart rate to 60 to 80 beats per minute or less and reduction of blood pressure to 120 mm Hg or less (systolic) with IV β-blockers
6. Type A dissection (and intramural hemorrhage and penetrating atherosclerotic ulcer of ascending aorta) is typically managed surgically
7. Type B dissection (and intramural hemorrhage and penetrating atherosclerotic ulcer of descending aorta) can be managed conservatively (treatment of hypertension) or surgically, depending on clinical findings
8. Patients with acute aortic syndrome need lifelong antihypertensive therapy and surveillance aortic imaging

Pitfalls

• Always consider aortic dissection as a cause of chest pain, particularly for pain that radiates to upper back or abdomen or that is described as tearing or ripping
• Missed or delayed diagnosis is usually due to an incorrect working diagnosis of acute coronary syndrome ²
  • Patients with an intermediate-risk profile for acute aortic syndrome who are being evaluated for a possible acute coronary syndrome (but without clear-cut ST-elevation myocardial infarction) should have aortic imaging before being given antiplatelet and antithrombin agents ³
  • Myocardial infarction can coexist with aortic dissection
• CT, MRI, and transesophageal echocardiography are all accurate diagnostic modalities, but false-negative results do occur
  • Diagnosis cannot be excluded definitively on the basis of results from a single imaging study. If first aortic imaging findings are negative, strongly consider obtaining a second imaging study if there is high clinical suspicion

Terminology

Clinical Clarification

• Acute aortic syndrome comprises several life-threatening aortic conditions that present similarly but have differing pathologic, demographic, and survival characteristics ^4 5
• Includes aortic dissection, intramural hematoma, and penetrating atherosclerotic ulcer ⁴⁶
  • Aortic dissection: disruption of medial layer provoked by intramural bleeding, usually from an intimal tear. This results in separation of aortic wall layers and subsequent formation of true lumen and false lumen with or without communication ⁷
    • Most common type of acute aortic syndrome ⁴
  • Aortic intramural hematoma: hematoma develops in media of aortic wall in absence of false lumen and intimal tear ⁷ (definition is controversial; intramural hematoma may be aortic dissection with thrombosis of false lumen) ⁴
    • Accounts for up to 25% of cases of acute aortic syndrome
  • Penetrating atherosclerotic ulcer: ulceration of an aortic atherosclerotic plaque penetrating through internal elastic lamina into media; ⁷ localized to descending thoracic aorta in more than 90% of cases ³
    • Accounts for 2% to 7% of cases of acute aortic syndrome ⁷

Classification

• Aortic dissection: classification based on anatomy
  • Stanford classification ⁵
    • Type A ⁸
      • Dissection originates in root of aorta or upper ascending aorta; may extend into abdominal aorta
      • Accounts for approximately two-thirds of acute dissections
      • Virtually all require surgical correction, and in-hospital mortality ranges from 22% to 47%
    • Type B ⁸
      • Dissection is confined to descending aorta
      • Accounts for approximately one-third of acute dissections
      • Most are uncomplicated type B dissections, which can be managed medically with very low in-hospital mortality; almost all progress to chronic phase
      • Complicated type B dissections require urgent endovascular or surgical repair for aortic rupture, rapid expansion, or branch vessel malperfusion
  • DeBakey classification ⁴
    • Type I
      • Dissection originates in ascending aorta and propagates distally at least to aortic arch and typically to descending aorta
      • 90% of type A dissections ⁸
    • Type II
      • Dissection originates in and is confined to ascending aorta
      • 10% of type A dissections ⁸
    • Type III
      • Dissection originates in descending aorta and propagates distally

Diagnosis

Clinical Presentation

• Classification systems for aortic dissection. – Systems classify the dissection by acuity, site/extent, and pathophysiology.From Kawabori M et al: Acute aortic syndrome: a systems approach to a time-critical disease. Best Pract Res Clin Anaesthesiol. 30(3):271-81, 2016, Figure 1.

History

• Earlier medical history is significant; known aortic aneurysm, congenital heart disease, connective tissue disease, hypertension, and previous surgical or catheter-based cardiac procedure increase likelihood of acute aortic syndrome
• Seek history of stimulant drug use (eg, cocaine, methylenedioxymethamphetamine, methamphetamine)
• Symptom onset is sudden and rapid
• Clinical symptoms do not allow distinction between acute aortic syndrome types and other acute pathologic conditions
• For all types of acute aortic syndrome, broad range of nonspecific symptoms is possible, depending on location of dissection, degree of extension, presence of vascular branch (and end-organ) involvement, and presence of rupture
  • Aortic dissection
    • Symptomatic in 80% to 90% of cases ⁴
    • Sudden-onset severe chest pain is first symptom
      • Most patients describe pain as sharp and knife-like; others describe it as tearing or ripping ^3 9
      • Abruptness of onset is a specific characteristic (84%) ⁹
    • Location of pain may reflect site of origin of intimal tear
      • Typically anterior chest pain in type A (80% of cases), but 47% of patients also report back pain ^3 10
      • Begins as back pain in type B (64% of cases), but presentation with chest and/or abdominal pain also occurs ³
      • Pain may be migratory as dissection extends
    • May present with syncope (15% with type A; less than 5% with type B) ⁷
    • Other symptoms reflect ischemic end-organ dysfunction caused by extension of dissection to branch arteries
      • Diffuse abdominal pain is present in about 21% of type A cases and about 43% of type B cases ³
      • Flank pain and decreased urine output are likely if renal artery is involved in dissection
      • Dyspnea and orthopnea are likely if ischemic congestive heart failure is present or if left ventricular failure results from aortic rupture into left atrium
      • Symptoms of stroke are likely if carotid artery is involved
      • Lower extremity numbness and pain are likely if dissection extends to iliac arteries (with paraplegia if spinal arteries are not perfused)
      • Rarely, hemoptysis is caused by rupture into tracheobronchial tree
      • Rarely, hematemesis is caused by rupture into esophagus
  • Intramural hematoma
    • Patient is more likely to be older and more likely to be female than a patient with aortic dissection ⁴
    • Presents with pain similar to that of aortic dissection ⁴
    • Less likely to present with neurologic symptoms
  • Penetrating atherosclerotic ulcer ¹¹
    • Patient is more likely to be an older male individual and more likely to have known coronary artery disease or risk factors for atherosclerotic disease than a patient with aortic dissection ⁷
    • Presents with pain similar to that of aortic dissection ^3 4
    • Rarely presents with symptoms of dissection-related lack of organ perfusion

Physical examination

• May appear anxious and diaphoretic
• Blood pressure may be high, normal, or low ¹²
  • Measure blood pressure in both arms; higher reading will be used to determine blood pressure management plan ³
  • Type A dissection
    • Hypotension in about 25% ⁹
    • Hypertension in about 35% ⁹
  • Type B dissection
    • Hypertension in about 70% ⁹
• Cardiopulmonary examination
  • Pulse deficit (apical versus radial) is present in about 18% of type A cases and about 9% of type B cases ⁹
  • Pressure differential (interarm blood pressure difference) greater than 20 mm Hg is suggestive (although studies differ on its significance) ¹⁰
    • Compare carotid and femoral arteries
  • Aortic insufficiency murmur (early diastolic decrescendo murmur heard best in the left third intercostal space) is present in about 44% of type A cases and about 12% of type B cases ⁹
  • Signs of congestive heart failure (eg, rales, S₃ gallop) are present in about 9% of type A cases and about 3% of type B cases ⁹
  • Cool, pale lower extremities with decreased or absent pulses are likely if iliac and femoral arteries are involved
• Neurologic examination
  • Signs of stroke are present in about 6% of type A cases and about 2% of type B cases ⁹
  • Hemiparesis is likely if spinal artery malperfusion is present
  • Hoarseness (from laryngeal nerve compression) may occur

Causes and Risk Factors

Causes

• Often multifactorial; most common factors include uncontrolled hypertension and inherited susceptibility ⁵
  • Hypertension
    • Elevated systolic pressure can propagate an aortic dissection
    • Physical exertion or emotional stress is direct precursor of pain in two-thirds of aortic dissection events; both involve acute changes in blood pressure ¹²
  • Genetic syndromes
    • Congenital connective tissue diseases (associated with abnormalities of aortic media)
      • Marfan syndrome ¹³
        • Autosomal dominant genetic disorder characterized by weakness of connective tissues (which endangers heart and blood vessels)
        • Aortic dissection occurs in 15% to 50% of patients with Marfan syndrome who are younger than 40 years ¹²
      • Ehlers-Danlos syndrome type IV ¹⁴
        • Autosomal dominant genetic disorder characterized by skin hyperextensibility, joint hypermobility, and tissue fragility (which endangers heart and blood vessels)
        • Arterial complications are the leading cause of death
      • Loeys-Dietz syndrome
        • Autosomal dominant genetic disorder characterized by arterial tortuosity, wide-set eyes, cleft palate, and aortic dissection and aneurysm
    • Other familial thoracic aneurysm and dissection syndromes
      • Familial Marfan-like habitus
      • Familial ectopia lentis
      • Familial thoracic aortic aneurysm and dissection
      • Erdheim cystic medial necrosis
      • Congenital contractural arachnodactyly
    • Turner syndrome
      • Phenotypic female with 45,X karyotype
      • May have associated bicuspid aortic valve and/or aortic coarctation

Risk factors and/or associations ¹⁵

Age

• Incidence rises with age; mean age at presentation is 63 years ⁹
• Age at presentation is often lower in patients with Marfan syndrome, bicuspid aortic valve, or history of previous aortic surgery ³
• Case reports of aortic dissection in children as young as 3 years ³

Sex

• More than two-thirds of aortic dissection events occur in male individuals ¹²
• Female individuals have higher mortality rates from aortic dissection than male individuals ¹⁶

Genetics

• Marfan syndrome: heterozygous mutation in the FBN1 gene (fibrillin 1; OMIM *134797) ¹⁷ on chromosome band 15q21 (phenotype is OMIM #154700) ¹⁸
• Ehlers-Danlos syndrome type IV: heterozygous mutation in the COL3A1 gene (collagen type III alpha 1 chain; OMIM *120180) ¹⁹ on chromosome band 2q32 (phenotype is OMIM #130050) ²⁰
• Loeys-Dietz syndrome type 2: heterozygous mutation in the TGFBR2 gene (transforming growth factor beta receptor 2; OMIM *190182) ²¹ on chromosome band 3p22 (phenotype is OMIM #610168) ²²

Other risk factors/associations

• Congenital cardiovascular malformations
  • Bicuspid aortic valve
    • Affects 1% to 2% of general population ⁷
    • Confers increased risk of aortic dissection; has been described in the range of 5- to 10-fold increased risk, although the true multiple is uncertain ^23 24
  • Aortic coarctation
    • Significantly increases risk of aortic dissection, particularly when in association with bicuspid aortic valve (odds ratio, 4.7) ¹²
• Preexisting thoracic aortic aneurysm ³
  • Aortic dissection is frequently superimposed on thoracic aortic aneurysms
• Previous cardiac interventions
  • Aortic dissection is a rare but dangerous complication of cardiac surgery and cardiac catheterization ²⁵
  • Data from International Registry of Acute Aortic Dissection show that about 4% of cases were a complication of these interventions ⁹
• Blunt chest trauma ³
  • High-energy deceleration injury is common mechanism
• Stimulant use (eg, cocaine, methamphetamine) ³
  • Associated with hypertension and hyperdynamic state
• Pregnancy
  • Marfan syndrome is present in up to half of pregnancy-associated cases of aortic dissection ¹³
  • Aortic dissection usually occurs in third trimester ¹³
• Acquired inflammatory vasculitides ³
  • May cause weakening of aortic wall
    • Giant cell arteritis
    • Takayasu arteritis
    • Behçet disease

Diagnostic Procedures

• Type A aortic dissection on CT. – A, Axial image from contrast-enhanced CT. Dissection flap (arrow) extends from aortic root to distal abdominal aorta. B, Coronal multiplanar reformation from another patient with involvement of aortic root (arrow), which puts this patient at risk for rupture into pericardial sac and resultant cardiac tamponade or aortic insufficiency, as well as extension of dissection flap into coronary arteries.From Scheske JA et al: Computed tomography angiography of the thoracic aorta. Radiol Clin North Am. 54(1):13-33, 2016, Figure 8.
• Type B aortic dissection on CT. – Axial contrast-enhanced CT. Dissection flap (solid arrow) is isolated to descending thoracic aorta. Focal low-density thrombus is present in beaked margin of false lumen (dashed arrow), which helps distinguish false lumen from true lumen. As in most cases, false lumen is larger than true lumen.From Scheske JA et al: Computed tomography angiography of the thoracic aorta. Radiol Clin North Am. 54(1):13-33, 2016, Figure 9.

Primary diagnostic tools

• History and physical examination findings suggest the diagnosis, but a high index of suspicion is necessary, given overlap with more common conditions ⁵
  • Best predictors are:
    • Abruptness of chest pain
    • Tearing or ripping quality of pain with migration
    • History of hypertension
    • Pulse deficit
    • Focal neurologic deficits
  • Consider risk scoring to determine pretest probability of acute aortic syndrome to direct testing strategy ²⁶
    • 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines ³ recommend the use of an algorithm that includes initial evaluation using ADD-RS (aortic dissection detection risk score); however, a 2015 clinical policy from the American College of Emergency Physicians ¹⁰ notes several limitations to this score, in particular, that it was found to be insufficient at excluding the diagnosis of aortic dissection
      • High-risk history includes known aortic aneurysm or aortic valve disease, connective tissue diseases (eg, Marfan syndrome), familial or genetic aortic aneurysm/dissection syndromes, and recent surgical manipulation of aorta (surgical or catheter-based)
      • High-risk pain features and physical signs include:
        • Abrupt onset of severe sharp or ripping chest/back/abdominal pain
        • Presence of pulse deficit
        • Blood pressure differential of more than 20 mm Hg between limbs
        • New murmur of aortic insufficiency
        • New focal neurologic deficit
      • Risk category: ³
        • 2 or more high-risk features: high risk
        • 1 high-risk feature: intermediate risk
        • 0 high-risk features: low risk
• Obtain imaging based on risk group ³⁷
  • Obtain urgent definitive aortic imaging (without waiting for chest radiograph) for all high-risk patients; if first aortic image finding is negative, obtain second imaging study for all high-risk patients or when suspicion is otherwise high ²⁷
    • CT angiography is diagnostic test of choice for most patients with high-risk features ^3 28
    • Transesophageal echocardiography can be performed at bedside for hemodynamically unstable patients with suspected type A disease for whom CT is not feasible ²⁷
      • Transthoracic echocardiography has lower sensitivity for identifying acute aortic syndromes than other modalities and is not considered definitive imaging
    • Magnetic resonance angiography is an additional option, if immediately available ²⁷
  • Order chest radiograph for all patients who are at low or intermediate risk, primarily to identify other causes of chest pain, which may obviate the need for definitive aortic imaging ²⁷
    • If dilated aorta or widened mediastinum is seen, proceed to definitive aortic imaging; chest radiograph alone is not sufficiently sensitive or specific for diagnosis of acute aortic syndrome
• Consider D-dimer testing, although do not use a specific result to direct further testing or treatment ²⁹
  • European Society of Cardiology guidelines ⁷ recommend obtaining D-dimer level; a negative result for a patient with pretest low-risk status decreases likelihood of aortic dissection ³⁰
  • American College of Emergency Physicians guidelines ¹⁰ and 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines ³ do not recommend routine D-dimer testing
• Additional testing is not diagnostic for acute aortic syndrome, but it is directed at differential diagnosis, identification of complications, and expediting time to surgery (for high-risk patients)
  • ECG and troponin levels ³
  • European Society of Cardiology guidelines ⁷ suggest the following be obtained routinely: CBC and levels of creatinine, AST, ALT, C-reactive protein, procalcitonin, lactate, and blood gases
  • Obtain presurgical laboratory testing for all high-risk patients, including CBC, coagulation tests, serum chemistry, and blood type and screen ³

Laboratory

Imaging

Other diagnostic tools

Differential Diagnosis

Most common ¹⁵

• Acute coronary syndrome (Related: Acute Coronary Syndromes)
  • Chest pain is usually the primary symptom in both scenarios; it is more likely to be of very abrupt onset and described as knife-like or tearing with acute aortic syndrome
  • Pulse differential and interarm blood pressure differential are unlikely in myocardial infarction
  • ECG may not always be helpful. Dissection-related coronary artery malperfusion may present with ECG changes that are indistinguishable from those of primary myocardial ischemia ³
  • Differentiate with immediate definitive aortic imaging (usually CT angiography) if patient is at intermediate or high risk for acute aortic syndrome
  • Myocardial infarction can coexist with aortic dissection, often as a complication thereof
• Pulmonary embolism (Related: Pulmonary Embolism)
  • May present with similar symptoms (eg, chest pain, dyspnea, syncope), although character of pain is more likely to be pleuritic with pulmonary embolism. Dyspnea may be a prominent symptom with pulmonary embolism
  • D-dimer level is elevated in both conditions; it typically rises faster (within first few hours) with aortic dissection, but it is not diagnostically helpful for differentiation
  • Differentiate with CT angiography
• Pneumothorax
  • Usually presents with dyspnea and sudden onset of ipsilateral chest pain described most commonly as pleuritic rather than tearing
  • Physical examination may suggest the diagnosis, with ipsilateral decreased breath sounds, absence of tactile fremitus, and hyperresonance to percussion; cyanosis, hypotension, and tracheal deviation are usually present in tension pneumothorax
  • Differentiate with posteroanterior chest radiograph, which shows loss of lung markings in periphery and a pleural line that runs parallel to chest wall
• Acute mesenteric ischemia (Related: Acute Mesenteric Ischemia)
  • Usually presents with acute onset of severe periumbilical pain disproportionate to physical examination findings, as opposed to pain of acute aortic dissection, which begins in chest and back and involves radiation of pain as dissection progresses
  • Patients with both conditions often have known cardiovascular disease or risk factors for cardiovascular disease
  • May occur as isolated event (usually due to thrombosis or emboli) or may occur secondary to aortic dissection extending to arteries branching off of the abdominal aorta
  • Differentiate the 2 conditions with CT angiography, imaging the entire abdominal aorta and all abdominal branch arteries
• Perforated peptic ulcer (Related: Peptic Ulcer Disease)
  • Presents with pain typically limited to abdomen in contrast to pain of acute aortic syndrome, which usually begins in chest and/or back
  • Patients with perforated peptic ulcer usually have rigid abdomen and remain motionless with pain; those with aortic dissection usually do not have rigid abdomen and often writhe in pain
  • Erect chest radiograph shows subdiaphragmatic air in perforated peptic ulcer
  • Differentiate acute aortic syndrome from perforated peptic ulcer with CT angiography if there is diagnostic uncertainty

Treatment

Goals

• In acute phase, decrease stress on aortic wall by reducing heart rate and blood pressure ³⁶³⁷
  • Reduce heart rate to 60 to 80 beats per minute or less (using β-blocker or nondihydropyridine calcium channel blocker) ²⁷
  • Reduce blood pressure to below 120 mm Hg (systolic) or to lowest blood pressure that maintains adequate end-organ perfusion (using vasodilators) ²⁷
  • Manage pain
• Correctly categorize type of acute aortic syndrome and treat appropriately to prevent propagation of dissection and other life-threatening complications

Disposition

Admission criteria

Criteria for ICU admission

• If not taken directly to surgery from emergency department, patients with acute aortic syndrome are admitted to ICU for the following: ³⁶
  • Intensive monitoring of vital signs
  • IV medication delivery
  • Preparatory staging for surgery or interventional radiology procedure

Recommendations for specialist referral ¹

• Obtain immediate urgent consult with cardiovascular surgeon upon diagnosis of acute aortic syndrome

Treatment Options

For all patients (with any cause, whether type A or B dissection, intramural hemorrhage, or penetrating atherosclerotic ulcer)

• Manage blood pressure, heart rate, and pain
  • If heart rate and/or blood pressure are more than 60 to 80 beats per minute or 120 mm Hg or more, respectively), reduce and maintain below these thresholds ²⁷
    • Begin therapy with IV β-blocker (eg, esmolol, labetalol, metoprolol) to reduce heart rate and blood pressure without causing reflex tachycardia ³
      • For β-blocker–intolerant patients, use nondihydropyridine calcium channel blockers (verapamil or diltiazem) ³⁸
        • Nondihydropyridine calcium channel blockers are not recommended for patients with heart failure with reduced ejection fraction due to negative inotropic effects ³⁹
      • Use these agents cautiously in the setting of acute aortic insufficiency, as compensatory tachycardia will be blocked ³
    • Add IV vasodilator if blood pressure goal is not achieved with β-blocker alone ²⁷
      • Options include sodium nitroprusside (most commonly used), ACE inhibitor, or dihydropyridine calcium channel blocker (eg, nicardipine, clevidipine)
      • Avoid administering before starting β-blockers or calcium channel blockers, as it may cause compensatory tachycardia ²⁷
  • If patient is hypotensive or in shock
    • Review imaging for causes (eg, contained rupture, pericardial tamponade, acute aortic insufficiency)
    • Shock usually mandates immediate operative intervention ²⁷
    • Resuscitation with fluid boluses with or without IV vasopressors; avoid inotropes, if possible, because they increase stress on aortic wall ³
    • For patients with hemopericardium and cardiac tamponade who cannot survive until surgery, consider performing pericardiocentesis, withdrawing just enough fluid to restore perfusion ³
  • Manage pain with IV opioids ²⁷
    • This will also help to control blood pressure and heart rate
• Determine definitive therapy (surgical versus medical) ²³⁶
  • Type A aortic dissection ²⁷
    • Surgical emergency
    • Proceed to surgery or expedite transfer to more appropriate facility; time intervals between symptom onset, diagnosis, and surgery can significantly affect survival ²⁷⁴⁰
      • Mortality rate of acute type A aortic dissection is 1% per hour of delay until surgery ²⁷
      • Patients presenting with clinical manifestations of shock, neurologic deficits, malperfusion, or myocardial ischemia require immediate consideration for surgical repair
      • Surgical intervention corrects aortic regurgitation and myocardial ischemia, reestablishes flow to malperfused vessels, and removes immediate risk of aortic rupture or tamponade ²⁷
  • Type B dissection ²⁷⁴¹⁴²
    • Complicated type B dissection
      • Surgical or endovascular intervention indicated for type B dissections associated with any of the following:
        • Uncontrolled hypertension
        • Refractory pain
        • Aortic rupture
        • Hypotension or shock
        • Malperfusion of limbs or viscera
      • Thoracic endovascular aortic repair has largely replaced open repair, providing anatomy is favorable, owing to lower morbidity and mortality rates ^27 43 44
    • Uncomplicated type B dissection
      • Most uncomplicated cases are managed medically to maintain heart rate and blood pressure goals ²⁷
      • Some surgeons recommend early endovascular aortic repair for uncomplicated type B dissection, particularly for patients with high-risk features on imaging, as it may delay disease progression and reduce risk of subsequent complications ²⁷⁴⁵⁴⁶
        • Optimal timing of intervention has not been established
      • Beyond the acute phase (ie, chronic aortic dissection), management consists of: ⁸
        • Lifelong antihypertensive therapy to maintain heart rate and systemic blood pressure goals
          • Transition from IV therapy to oral route
          • Regimens that include β-blockers, ACE inhibitors and angiotensin receptor blockers ⁴⁷
        • Statin therapy may be beneficial
          • Associated with greater long-term survival for medically managed patients (reducing both cardiovascular mortality and aortic-related mortality) ⁴⁸
        • Lifelong surveillance with aortic imaging
        • Aortic repair once the dissected aorta becomes significantly aneurysmal
          • Repair is indicated for chronic thoracic and thoracoabdominal aortic dissections with a maximal aortic diameter of 6.0 cm for patients without connective tissue disease ⁸
          • Patients with connective tissue disease and those with a family history may warrant earlier intervention at aortic diameter of 5.0 to 5.5 cm or even lower in some specific heritable thoracic aortic aneurysm cases ²⁷
          • Rapid growth of the aortic root or ascending aortic aneurysm growth is also an indication for repair ⁸²⁷
            • Defined as 0.5 cm or more in 1 year or 0.3 cm or more per year for 2 consecutive years for those with sporadic aneurysms or 0.3 cm or more in 1 year for those with heritable thoracic aortic disease or bicuspid aortic valve
          • Open surgical repair is the main repair method; however, thoracic endovascular aortic repair is reasonable for patients with indications for intervention who are at high risk for complications of open repair due to comorbidities ^8 44
  • Intramural hemorrhage
    • Intramural hemorrhage involving the ascending aorta (type A intramural hemorrhage) has high mortality with medical treatment alone; treatment is usually surgical ³
      • Timing of intervention varies: urgent surgery in the case of complicated type A intramural hemorrhage, planned surgery within 24 hours or within a few days, expectant medical management with surgical intervention only for complications or disease progression ²⁷
    • Intramural hemorrhage involving the descending aorta (type B intramural hemorrhage) is managed medically (blood pressure control) in many cases, but surgery or endovascular repair is increasingly being used ⁴⁹
  • Penetrating atherosclerotic ulcer
    • Generally requires early surgical intervention if associated with persistent pain, hemodynamic instability, or high-risk imaging features (large diameter or depth, significant growth on surveillance imaging, or increasing pleural effusion) ^27 49 50

Drug therapy

• Blood pressure and heart rate management
  • β-Blockers
    • Esmolol
      • Esmolol Hydrochloride Solution for injection; Adults: 500 to 1,000 mcg/kg IV over 1 minute, then 50 mcg/kg/minute continuous IV infusion, initially. Repeat bolus and titrate by 50 mcg/kg/minute until goal blood pressure is attained. Max: 200 mcg/kg/minute.
    • Labetalol
      • Labetalol Hydrochloride Solution for injection; Adults: 10 to 20 mg IV, then 20 to 80 mg IV every 10 to 30 minutes until goal blood pressure is attained. Max cumulative dose: 300 mg.
    • Metoprolol
      • Metoprolol Tartrate Solution for injection; Adults: 2.5 to 5 mg IV every 5 minutes as needed up to 15 mg.
  • Sodium nitroprusside
    • Used in conjunction with β-blocker to offset tachycardia caused by nitroprusside
      • Sodium Nitroprusside Solution for injection; Adults: 0.3 to 0.5 mcg/kg/minute continuous IV infusion, initially. Titrate by 0.5 mcg/kg/minute every 5 minutes until desired effect or blood pressure cannot be further reduced without compromising organ perfusion. Max: 10 mcg/kg/minute for 10 minutes.
  • Dihydropyridine calcium channel blockers (indicated as vasodilator for hypertension)
    • Clevidipine
      • Clevidipine Emulsion for injection; Adults: 1 to 2 mg/hour continuous IV infusion, initially. Double dose every 90 seconds until the blood pressure approaches goal, then increase by less than double every 5 to 10 minutes as needed. Max: 32 mg/hour or 1,000 mL/24 hours due to lipid load restrictions. Max duration: 72 hours.
    • Nicardipine
      • Nicardipine Hydrochloride Solution for injection; Adults: 5 mg/hour continuous IV infusion, initially. Titrate by 2.5 mg/hour every 5 to 15 minutes until goal blood pressure is attained. Max: 15 mg/hour. Reduce to 3 mg/hour after response achieved.
  • ACE inhibitors
    • Enalaprilat
      • Enalaprilat Solution for injection; Adults: 0.625 to 1.25 mg IV every 6 hours, initially; may increase dose up to 5 mg IV every 6 hours.

Nondrug and supportive care

Advise all patients with aortic disease to avoid competitive and body contact sports and strenuous activities that would require the Valsalva maneuver (eg, isometric heavy weightlifting, shoveling snow, chopping wood) ⁷

• Low resistance exercise that avoids straining is generally considered acceptable except in cases of severe aortic dilatation

Procedures

Surgery

General explanation

• Gold standard intervention
• Dissected aortic section is removed and replaced with synthetic graft
• Some patients may require additional immediate reconstruction owing to nature of dissection
  • Aortic valve revision or replacement
  • Coronary artery bypass grafting
• Techniques such as hypothermic circulatory arrest and perfusion of the head vessels are often used during surgery

Indication

• Type A dissection
• Complicated type B dissection that cannot be managed medically and for which an endovascular approach is not appropriate

Complications

• Operative mortality rate is now less than 18% for type A dissection ^51 52

Thoracic endovascular aortic aneurysm repair ^7 36

General explanation

• Stent is inserted through catheters inserted into femoral artery
• Balloon is inserted, covering primary entry tear and restoring blood flow into true lumen
• Prevents further enlargement and eventual aortic rupture

Indication

• Preferred approach for most patients with type B dissection ⁴³, penetrating atherosclerotic ulcer of descending aorta, or intramural hematoma of descending aorta

Complications

• Access site complications are uncommon ⁷
• Paraparesis/paraplegia occurs in 0.8% to 1.9%, and stroke occurs in 2.1% to 3.5% (lower risk than for open surgery) ⁷

Pericardiocentesis ⁵³

General explanation

• Aspiration of pericardial fluid, ideally under real-time ultrasonographic or fluoroscopic guidance. In an emergency setting, performed without such guidance
• Patient is placed in supine or semirecumbent position with continuous ECG monitoring; anesthesia is usually local
• Spinal needle is advanced through subxiphoid skin toward left shoulder or suprasternal notch. Needle is further advanced blindly (in emergency) or with ECG connector attached to the hub while negative pressure is applied to syringe. A pop is usually felt when puncture of pericardium occurs
• When blood or pericardial fluid is aspirated or cardiac pulsations are felt, a syringe is attached to the needle and pericardial fluid is withdrawn
  • ECG changes suggesting pericardial penetration (increased P-wave amplitude, ST-segment elevation, or ectopic beats) indicate the need to withdraw needle slightly in 1- to 2-mm increments until the changes disappear

Indication

• Cardiac tamponade

Contraindications

• Anticoagulation is relative contraindication in life-threatening situations

Complications

• Myocardial puncture with hemopericardium
• Coronary artery laceration
• Bradyarrhythmias and tachyarrhythmias
• Pneumothorax or hemothorax
• Hypovolemic hypotension from removing large volumes of fluid
• Recurrence (up to 70% using blind technique)
• Cardiac arrest

Interpretation of results

• When procedure is done without sonographic or fluoroscopic guidance, false-negative aspiration rates are as high as 80% (usually owing to clotted blood in pericardial space or failure to enter pericardial space)

Special populations

• Pregnancy ¹³
  • Survival rate for aortic dissection during pregnancy is 20% to 30%
  • Aortic dissection is most common in third trimester or postpartum period
  • Marfan syndrome is present in up to half of pregnancy-associated cases of aortic dissection
  • Beyond 30 weeks of gestation, emergency cesarean delivery and subsequent cardiac surgery may be the best option to save the lives of both infant and child bearer
  • Refer patients with known aortic disease or at risk for aortic disease, including those with associated genetic syndromes or disease, for preconception and genetics counseling

Monitoring

• After type A or B dissection and intramural hematoma ³
  • Follow-up aortic imaging should be obtained at 1, 3, 6, and 12 months and then annually thereafter ^8 49
  • Use the same imaging modality (CT or MRI) at the same institution if possible, so that similar images of matching anatomical segments can be compared side by side
  • If stable over long term, consider switch from CT to MRI to decrease radiation exposure

Complications and Prognosis

Complications

• Death
  • Untreated acute type A dissection has a mortality rate of about 1% to 2% per hour initially ^40 54
  • For acute type B dissection, in-hospital mortality is 10.6% ⁵⁵
• Myocardial infarction (Related: Acute Coronary Syndromes)⁵⁶
  • Can occur from aortic dissection involvement of aortic arch, secondary to massive blood loss, or as a complication of treatment
• Aortic valve regurgitation or rupture ⁵⁶
  • Can be a complication of aortic dissection itself or of surgical treatment
  • Physical signs include early diastolic decrescendo murmur, heard best in left third intercostal space and radiating to left sternal border
• Cardiac tamponade ⁵⁶
  • Aortic dissection can cause rupture into lining around heart
  • Physical signs include jugular venous distention, pulsus paradoxus, and distant heart sounds on auscultation
• Stroke or other neurologic complications
  • Neurologic complications, including paraparesis, occur in about 14% ⁵⁷ to 17% ³ of patients with dissection
• Organ and peripheral damage ⁵⁶
  • Kidney failure (Related: Acute Kidney Injury)
    • Can be caused by arterial embolism, by hypotensive state, or directly by propagation of aortic dissection to renal artery
  • Mesenteric ischemia
    • Can be caused by arterial embolism, by hypotensive state, or directly by propagation of aortic dissection to visceral arteries
  • Limb ischemia
    • Can be caused by arterial embolism, by hypotensive state, or directly by propagation of aortic dissection to iliac or femoral arteries
• Development of a secondary aortic dissection after surgical or endovascular repair
  • Typically develops from a site just distal to surgical or endovascular graft
• Progressive aortic disease
  • Progressive disease, including aneurysmal degeneration, occurs in 30% of medically managed patients with uncomplicated type B dissection ⁵⁸

Prognosis

• When aortic disease is detected early and treated promptly, chances of survival greatly improve ³⁶³⁷
  • Aortic dissection
    • 10-year survival, independent of type of dissection, is 35% to 70% ⁵⁹
    • Reoperation is required for up to one-third of patients ⁵⁹
    • Type A dissection
      • Operative mortality has declined; it is now less than 18% ⁵²
      • Lower operative mortality in hospitals with high case volume versus those with low case volume ⁷
      • Patients treated medically have mortality of 50% ⁵⁹
    • Type B dissection
      • Patients with medically treated uncomplicated type B dissection have 10% mortality over 5 years ⁵⁸
        • Morbidity, including aneurysmal degeneration, occurs in 30% ⁵⁸
      • Use of thoracic endovascular aortic aneurysm repair for management of complicated type B dissection has greatly increased early survival for these patients ⁴²
  • Intramural hematoma
    • May regress, expand, progress to classic dissection, or rupture
    • Reported mortality has varied by region
      • In 3 studies, early mortality with surgery (versus without surgery) was as follows: 0% (versus 80%), 8% (versus 55%), and 14% (versus 36%) ⁶⁰
      • However, another study primarily from Asian centers showed little difference in outcomes between surgical and medical treatment ⁶⁰
  • Penetrating atherosclerotic ulcer
    • 30-day mortality for surgical treatment is 21% versus 4% for medical treatment, but some case series have reported much higher mortality for medical management ⁶⁰
    • Patients with acute penetrating atherosclerotic ulcers are at high risk for aortic-related adverse events and clinically related adverse events within 30 days after onset ¹¹

Screening and Prevention

Screening

At-risk populations ¹⁵

• Patients with bicuspid aortic valve
• Patients with known genetic conditions
  • Marfan syndrome
  • Loeys-Dietz syndrome
  • Ehlers-Danlos syndrome type IV
  • Familial thoracic aortic aneurysm or dissection
  • Turner syndrome
• First-degree relatives of patients with aneurysms of the aortic root or ascending aorta or with aortic dissection ²⁷
• Recent aortic manipulation
• Aortic aneurysm

Screening tests

• Genetic testing and counseling ³
  • Recommended for:
    • First-degree relatives of a patient with known thoracic aortic aneurysm or dissection
    • First-degree relatives of a patient with known genetic mutation associated with thoracic aortic aneurysm or dissection; after genetic testing and counseling, only the relatives with the genetic mutation should undergo aortic imaging
• Aortic imaging and surveillance
  • Modalities include CT angiography of chest and abdomen with IV contrast, magnetic resonance angiography of chest and abdomen with IV contrast, magnetic resonance angiography of chest and abdomen without IV contrast, and chest radiography ⁶¹
  • Recommended for: ³
    • Patients with thoracic aortic aneurysm
    • Patients with bicuspid aortic valve
    • Patients with known genetic syndromes associated with thoracic aortic aneurysm and/or dissection
      • Marfan syndrome
        • Echocardiogram is recommended at time of diagnosis of Marfan syndrome to determine diameters of aortic root and ascending aorta ³
        • Repeat at 6-month intervals to determine rate of enlargement of aorta; if stable, can decrease to annual imaging ³
        • If maximal aortic diameter is 4.5 cm or more, or if aortic diameter shows significant growth from baseline, obtain additional imaging ³
      • Loeys-Dietz syndrome or another confirmed (non-Marfan) genetic mutation known to predispose to aortic aneurysms and aortic dissections
        • Complete aortic imaging at initial diagnosis ³
        • Repeat at 6-month intervals to determine if enlargement is occurring ³
        • Patients with Loeys-Dietz syndrome should have yearly imaging from cerebrovascular circulation to pelvis ³
      • Turner syndrome
        • Baseline imaging of heart and aorta for evidence of bicuspid aortic valve, coarctation of aorta, or dilation of ascending thoracic aorta ³
        • If initial imaging findings are normal and there are no risk factors for aortic dissection, obtain repeated imaging every 5 to 10 years or if otherwise clinically indicated ³
        • If abnormalities exist, obtain annual imaging or follow-up imaging ³
    • First-degree relatives of patients with thoracic aortic aneurysm and/or dissection to identify those with asymptomatic disease ³
      • Imaging of second-degree relatives is reasonable if 1 or more first-degree relatives of a patient with known thoracic aortic aneurysm and/or dissection are found to have thoracic aortic dilation, aneurysm, or dissection

Prevention

• Patients with known thoracic aortic disease: ⁸
  • Antihypertensive therapy
  • Restriction from contact sports and other strenuous activities
  • Lifelong surveillance with aortic imaging
  • Surgical intervention based on aortic caliber, growth rate, pathogenesis, and presence of symptoms ⁸
• Patients with Marfan syndrome or Ehlers-Danlos syndrome type IV: restriction from contact sports and other strenuous activities ^6 62
• Patients with Marfan syndrome: prophylactic elective replacement of aortic root may be an option ^6 63

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